We describe a patient with widespread skin lesions and circulating IgG autoantibodies to both type VII collagen and laminin 5. Although autoantibodies to type VII collagen belonged to IgG2, IgG3, and IgG4 subclasses, ...We describe a patient with widespread skin lesions and circulating IgG autoantibodies to both type VII collagen and laminin 5. Although autoantibodies to type VII collagen belonged to IgG2, IgG3, and IgG4 subclasses, laminin 5 was targeted exclusively by IgG3 autoantibodies. Interestingly, despite the presence of IgG3 autoantibodies, the patient’ s serum failed to fix complement to the dermoepidermal junction. In addition, these autoantibodies did not recruit and activate leukocytes or induce dermoepidermal separation in skin sectioned by cryostat. We report a most unusual case of an autoimmune subepidermal blistering with an exclusive IgG3 reactivity to laminin 5.展开更多
Background:Netherton syndrome (NS) is a rare autosomal re-cessive condition characterized by ichthyosi form erythroderma, trichorrhexis invaginata and atopi c manifestations. Confirming the diagnosis may be difficult ...Background:Netherton syndrome (NS) is a rare autosomal re-cessive condition characterized by ichthyosi form erythroderma, trichorrhexis invaginata and atopi c manifestations. Confirming the diagnosis may be difficult in the early stages. Mutations in the SPINK5 gene which encodes for the serine protease inhibitor LE KTI are associated with NS. These mutations create premature termination codons which result in absent or abnormal expression of LEKTI in patients with NS. Obje ctives:To investigate the expression of LEKTI in the skin of patients with NS i n comparison with normal controls and patients with other skin conditions, namel y atopic dermatitis, psoriasis and nonbullous ichthyosiform erythroderma. Method s:Immunohistochemistry was performed on skin sections from four patients with N S, four normal controls, four with atopic dermatitis, two with psoriasis and two with nonbullous ichthyosiform erythroderma, using a primary rabbit polyclonal a ntibody against LEKTI. Results:LEKTI was localized to the stratum granulosum in normal skin. All four skin sections from patients with NS showed absent or very reduced staining for LEKTI. Staining in the other disorders showed positive LEK TI expression in varying patterns. Conclusions:NS can be difficult to diagnose especially in the early stage, which can lead to inappropriate treatments partic ularly if it is misdiagnosed as atopic dermatitis. Immunohistochemistry of skin with an antibody against LEKTI is a potentially useful diagnostic test for NS.展开更多
文摘We describe a patient with widespread skin lesions and circulating IgG autoantibodies to both type VII collagen and laminin 5. Although autoantibodies to type VII collagen belonged to IgG2, IgG3, and IgG4 subclasses, laminin 5 was targeted exclusively by IgG3 autoantibodies. Interestingly, despite the presence of IgG3 autoantibodies, the patient’ s serum failed to fix complement to the dermoepidermal junction. In addition, these autoantibodies did not recruit and activate leukocytes or induce dermoepidermal separation in skin sectioned by cryostat. We report a most unusual case of an autoimmune subepidermal blistering with an exclusive IgG3 reactivity to laminin 5.
文摘Background:Netherton syndrome (NS) is a rare autosomal re-cessive condition characterized by ichthyosi form erythroderma, trichorrhexis invaginata and atopi c manifestations. Confirming the diagnosis may be difficult in the early stages. Mutations in the SPINK5 gene which encodes for the serine protease inhibitor LE KTI are associated with NS. These mutations create premature termination codons which result in absent or abnormal expression of LEKTI in patients with NS. Obje ctives:To investigate the expression of LEKTI in the skin of patients with NS i n comparison with normal controls and patients with other skin conditions, namel y atopic dermatitis, psoriasis and nonbullous ichthyosiform erythroderma. Method s:Immunohistochemistry was performed on skin sections from four patients with N S, four normal controls, four with atopic dermatitis, two with psoriasis and two with nonbullous ichthyosiform erythroderma, using a primary rabbit polyclonal a ntibody against LEKTI. Results:LEKTI was localized to the stratum granulosum in normal skin. All four skin sections from patients with NS showed absent or very reduced staining for LEKTI. Staining in the other disorders showed positive LEK TI expression in varying patterns. Conclusions:NS can be difficult to diagnose especially in the early stage, which can lead to inappropriate treatments partic ularly if it is misdiagnosed as atopic dermatitis. Immunohistochemistry of skin with an antibody against LEKTI is a potentially useful diagnostic test for NS.
